446 - Neuromyelitis Optica Spectrum Disorder: A Multifaceted Case Presentation

Case Diagnosis: Neuromyelitis optica spectrum disorder (NMOSD) with area postrema syndrome and cardiac dysregulation

Case Description: A 41-year-old male presented with a sudden loss of vision in his right eye lasting two days. Brain MRI revealed right optic neuritis, raising suspicion of demyelinating disease. Intravenous methylprednisolone treatment was administered, and cerebrospinal fluid analysis indicated positive antibodies. A diagnosis of NMOSD was established, necessitating ongoing steroid therapy. Two months later, the patient returned to the hospital with unrelenting vomiting and hiccups persisting for three days. A subsequent MRI revealed new T2 hyperintensities in the left area postrema, resulting in a diagnosis of area postrema syndrome. Treatment with intravenous immunoglobulin (IVIG) led to substantial symptom improvement. One month after that, the patient re-presented with right lower extremity weakness and two days of urinary retention, complicated by persistent tachycardia. Brain MRI revealed hypoattenuation affecting the diencephalon, cerebral white matter, and brainstem. Other potential causes of tachycardia were excluded. The patient received plasmapheresis and was subsequently admitted for acute rehabilitation.

Discussions: NMOSD is a rare autoimmune disorder characterized by multifocal inflammation in the central nervous system, primarily affecting the optic nerves. It displays a female predominance in adults, particularly in the AQP4-Ab-seropositive subgroup (up to a 9:1 ratio), who tend to experience more clinical attacks. In this instance, a male patient with AQP4-Ab-seropositive NMOSD presented not only with motor deficits but also with area postrema syndrome and cardiovascular dysregulation. An interdisciplinary approach was employed throughout the inpatient rehabilitation process, addressing physical, cognitive, and emotional aspects of care. Substantial progress was achieved during rehabilitation, resulting in improved functional capacity and a safe discharge home.

Conclusions: This case emphasizes the importance of recognizing potential complications associated with NMOSD, impacting both medical status and functional capacity. It also underscores the effectiveness of a multidisciplinary strategy in the management of NMOSD.